Tag Archives: pierre robin sequence

As an active blogger and Social Media participant, I choose to live my life out loud. In choosing to live my life in this manner, I open myself and my life up for comments and questions. Sometimes, these comments and questions hurt. Sometimes they are meant to cut. Other times, they are not meant to hurt but are instead posed with the best of intentions. The latter often catch me off guard. Such a situation occurred this past week. Instead of dashing off a quick and angry response, I called a friend and nearly ended up in tears as I described the situation to him. I let it sit over night as I thought about the best way to respond. Then I took to Twitter to vent about the situation. Once I took to Twitter, I realized I was not alone in my very justified reaction to the inquiry. Instead of a private response, I choose to handle this in a public manner. There are many other mothers of children with birth defects in this world and all of us battle the same thing deep down inside. All of us are consumed by guilt.

The birth of my second daughter at 35 weeks and 5 days occurred 42 hours after my first contraction. I pushed twice and her screams filled the air of the delivery room instantly as she emerged into my nurse’s waiting arms. Placed on my chest, she continued to scream and writhe about as most newborns do. At first glance, she appeared healthy. All fingers, all toes, you know, the important stuff. When she screamed however, her mouth gaped at the top where her palate should have been. I blinked and tried to check but blamed it on exhaustion. I tried to latch her onto my breast to nurse but it didn’t work. After several tries, on and off, her screaming, me almost in tears, we requested the Lactation Consultant.

The Lactation consultant came in, slipped on gloves, and swiped our 30 minute old daughter’s mouth. “She’s got a cleft.” A swarm of activity buzzed about our room and suddenly there I was, alone, in bed, freshly delivered and still numb from the epidural. No one to talk to, no one to explain to me what was going on. The thoughts started. I knew of a cleft. I knew it meant something was missing. But I didn’t know the cause. I didn’t know why. Then I thought. I thought some more. What had I done wrong?

Early in my pregnancy, I was unable to take prenatal vitamins because they induced severe nausea. Forced to choose between taking the vitamins or not eating, I chose to not take the vitamins. I even tried taking them at night but it was a no go. My depression from the birth of our first daughter also played into the decision to not discuss this nausea at length with my OB. Nausea continued well into the 6th month of my pregnancies. By the 6th month, though, I still was not taking my prenatals. In my depression delusional mind, I even wondered if it would truly affect my growing child’s well-being.

At six months pregnant, however, even if I HAD taken my prenatals, it wouldn’t have mattered. Most clefts form between 4-6 weeks, well before a woman is even aware of her pregnancy. Many clefts are even impossible to link to a specific cause. Our daughter’s specific cleft, a bilateral complete cleft of both hard and soft palate (meaning essentially, she had NO PALATE whatsover), was associated with a condition called Pierre Robin Sequence (pronounced Pea-air Roh-ban). Her jaw was also recessed, her tiny tongue was floppy, and her airway was narrow. In the 1920’s, PRS babies had a slim chance of survival. Today, however, the rate of survival is very high and surgery is available to correct these issues.

I was asked, several times, by several doctors, if I had taken my prenatal vitamins. I lied. Yes, I know I shouldn’t have lied. I should have been honest. But between depression, PTSD, and the guilt now whirring around in my head, rational behaviour escaped me. My partner didn’t even know I hadn’t taken my prenatals until I confessed while in labor with our son. (Hell of a time to confess, huh?)

Bottom line: I BLAMED MYSELF FOR MY DAUGHTER’S CLEFT.

Yes, rationally I know now I am not to blame. There is no family history of cleft. No associated genetic syndrome along with her PRS. It formed well before I could have done anything about it and even Mothers who take folic acid religiously still have a risk of giving birth to a child with a cleft. I know clefts are nearly impossible to see on a standard u/s unless you are looking for them specifically. Intellectually, rationally, I know all of this. and yet, the guilt consumes me. She grew inside me. She grew imperfectly. Logically I am to blame. If she is imperfect, there is something wrong with me. I failed my daughter before she was even born. I failed at motherhood a second time before I even held her. I FAILED.

Mothers of children with birth defects, with special needs know what I am talking about. We feel this every day. We fight like hell to not let this guilt eat at us. We fight against stigma, misinformation, judgment, and ignorance. We live with the stares, with the internal guilt which threatens to rip us apart every second of the day. We ferociously fight for our children so they may have a chance to live a normal life. A life of which they are completely worthy.

My daughter is nearly 6 years old now. She is beautiful. She is intelligent. She is determined, obstinate, and full of perseverance. She is happy. She is thriving. She is PERFECT. She is LOVED.

It doesn’t matter what I did or didn’t do all those years ago. I cannot go back in time to change anything which happened. Even if I could, I would not want to go back in time to do so. Because if I did, I wouldn’t have a daughter who has taught me more than anyone else in my life about the importance of hanging in there, fighting for even the simplest things (like speech, breathing, and eating), or that the most important thing in life is to be happy and keep others laughing right along with you.

Our daughter was 56 days old. She had spent just 15 days more at home than in the NICU at that point, having been born with a cleft palate, micrognathia, and glossotopsis. This is known as Pierre Robin Sequence. By the time we got her home, she had endured major surgery, been in a medically induced coma for a week, and had a feeding tube placed. More surgeries would be necessary to close the cleft of both her soft and hard palate. The cleft was complete and bilateral, meaning there was NOTHING up there but a gaping hole.

The day of her jaw surgery, I checked out. Curled up in the sleep room with Linkin Park’s Remix album and wanted to sink deep down into the chair. It was dark there. And safe. Oh so safe.

I cried, no, bawled, my body wracked with tears that I had muscle aches the next day. I wanted to leave her at the hospital. How the hell could this have happened to us? Why us? What the hell was He thinking? I pushed her away from the very beginning. Her cleft destroyed all of my expectations. Birth, breastfeed, go home. All of this in between NICU crap wasn’t in the plans. Formula wasn’t in the plan. abandonment just 30 minutes after a 2 day active labor wasn’t in the plans. My new daughter going to Atlanta without us at less than 24 hours old – SO not in the plans. My mom picking me up at the hospital – not in the plans. Our 23 month old daughter’s life being turned upside down – I felt guilty.

I didn’t take my pre-natal vitamins. Clefts can be a result of poor maternal diet, folic acid specifically. I had severe nausea and wasn’t able to eat most days. SO I didn’t take my vitamins. Ever. Looking back, depression flecked the entire pregnancy. And now this? I would have fared better in a ring with Mike Tyson.

The night of her birth, I woke up at 10pm to pee. I stood there and brushed my hair for 10 minutes. I didn’t see myself in the mirror. What I saw was a shell. I willed a spark to appear – but none did. Eventually I gave up and went back to bed, lying there, confused, exhausted, worried – slipping in and out of sleep only because physically I needed to collapse into bed!

The next day I yelled at our nurse when she tried to get us to sign consent forms for C to go to Atlanta. But she wasn’t supposed to go until later in the week. What do you mean this AFTERNOON? Where are you taking my baby? Why are we.. you can’t take her. You just can’t. you.just.can’t.

More hustle and bustle. In and out. Charlotte seems to spend the entire day away from me. I spend the entire day away from her. But at one point, I am in the bathroom and she’s in her bassinet in the room. I hear a door open. A man walks in and I freak out. He prays with me and leaves. I’m scared and go to the desk to ask that no one be allowed in the room unless they are on a list I’ve scribbled up. On the list are our parents. No one else, no one else. I am not in the mood for random strangers to stop by. (I think he went to our church)

By that afternoon, we meet the transport team. They seem nice enough. Chris has bought a little lamb to ride in the incubator looking rig with her. She’s healthy, they tell us. Oxygen sats are good, breathing is good, she’s healthy. She’ll be fine. We’ll meet your husband there. We sign the first of a slew of paperwork.

Chris hangs out with me after she leaves and eventually he has to leave too.

I pace in the hospital room when I am alone. Pace, pump, clean, pace, pump, clean.

I am a caged animal blaming myself for my daughter’s issues. I caused this by not taking my prenatal vitamins. This hell is all my fault. I did this to my poor baby girl.

Later that evening, my in-laws swing by with On the Border and my daughter. They stay for a few minutes. It’s painfully uncomfortable, perhaps a misconception on my part. After they leave, I voraciously eat my now cold food as I watch Nothing to Lose, my go to happy movie. It doesn’t work.

By now, Chris is in Atlanta. I call him around 10pm and cry so hard he can’t understand a damned word I’m saying. We hang up and I wail myself to sleep. If I had given birth via c-section, I am sure I would have torn stitches. Again, I wake up to pee in the middle of the night. Again, I stand and brush my hair. But this time I straighten up the already immaculate room as well before going back to bed.

The following morning, one of the OB’s from the practice offers me Prozac. I decline, saying that I want to see how far I can get on my own before I go jumping into meds. I’m stubborn like that. The OB I hated came by to tell me we were doing a great job and everything would be okay. I wanted to believe him.

Later that day, my mother picks me up. We pick up Allison and head home. I collapse. We manage to get a rental breast pump delivered that night (oh sweet relief as my milk has finally started to come in). I double pump in front of my mother using a t-shirt as cover. Eventually I give up on trying to hide the pumps.

That Friday I went to Atlanta to see her in the NICU. I’m heartbroken. I don’t want to be there. We’re not supposed to be there. What do I know about NICU babies? Why am I mother to one? Who the hell approved this script change? I didn’t.

Over the next few days, Chris and I spend some time together at his Uncle’s house as we ferry back and forth to the hospital. We talk about having another baby (see how far gone I was!) and I quietly wish we could leave her a the hospital but don’t tell him this for a couple of weeks.

That first week the feeding team wants to get Charlotte up to speed but she’s not cooperating. So the Plastic Surgeon suggests a jaw distraction which gets the feeding team pissed and puts us in the middle. We go to the garden at the hospital and I cry on Chris’ shoulder.

We decide to go with the surgery. At 9 days, she’s prepped and we leave her for surgery downstairs. I cry – again. His parents are there. I don’t want them to be but he needs support and I’ve chosen to respect that. I get the Mp3 player and disappear into the sleep room. I’m safe there. Very safe and lost in Linkin Park.

She comes back up in a medically induced coma. A machine breathes for her, she’s swollen, shiny, and tiny. But she’s had a good surgery. She made it through.

That afternoon, my husband calls the OB for me. I’m not doing well. We make an appointment for the next day. I made it 9 days, I tell them. I need something. I need help. I want to function because right now, right now I am not.

Right now, I’m brushing my hair, changing my pads, washing my hands, washing my pump parts, and it’s all very routine and necessary but it’s also very comforting. Very very comforting. I use the same bathrooms at the hospital. I use the same sinks at the NICU. I don’t stray outside my comfort zone. I kept to this routine the entire time she was in the hospital. I got edgy if it changed. At all.

Beginning of April I sprain my ankle as I get up from pumping. It’s the day we’re supposed to learn how to place an NG tube so we don’t have to have more surgery for a G-tube. I wrap up my ankle, bag up the ibuprofen and tylenol and go. There’s a grown up hospital across the street if it gets bad, I tell my husband.

I can’t place the NG tube. I officially suck, I tell myself. I suck. She’s angry at me because I suck. I can’t take care of my daughter. What the hell kind of mother am I? She’s my daughter. I should be able to do whatever I need to care for her. But I can’t. And so I have failed. Again.

We decide to go with a g-tube. It’ll be easier for me. I feel guilty for making her go through a surgery because it’ll be easier for me but easier for me means better for her. So that makes it okay, right? Right?

Right.

At 21 days old, she comes home. The ride home we can’t figure out how to get her pump to work. That night we can’t get it to work. I stay up with her because I can sleep the next day. Chris has to go to work. I don’t sleep well. I pump, I feed, I care for our 23 month old daughter and two dogs. A vicious cycle has begun.

Within two weeks, I ask for my meds to be upped. They’re not working. I’m stressed. My thoughts are getting more and more intense. They need to stop. The meds will make them stop. Make them stop. MAKE them stop.

Within three weeks, the thoughts are firing so rapidly at me I wonder if I’m in front of a death squad. I’m disgusted and repulsed. Pillows, visions of death, horrible deep dark secrets slam into me every few minutes. They’re like contractions on speed, really, waves that don’t ever seem to stop.

Our second daughter was born three and a half weeks early after 42 grueling hours of labor. I was not on medication during my pregnancy with her. 30 minutes after birth, the lactation consultant discovered she had a cleft palate. Within 24 hours, she was an hour away at a Children’s Hospital in the NICU while I recovered for another 48. Within 9 days, she had her first major surgery. Within 21 days, she had undergone two more surgical procedures. The first five months of her life saw a grand total of six surgeries. Since then she’s had two more. Our daughter will be four in March and has already had eight surgical procedures.

She drank from a special bottle when she was not being tube fed. I pumped exclusively for seven grueling months stopping only because my mental health depended on me making a very difficult decision. While she does not struggle with basic needs as much as she did, it still takes her longer than usual to chew because her bite is lopsided. You see, her teeth only meet when she grits them together at one pivotal side – the right side. Her speech is garbled. I can only understand 50 – 75% of my own daughter’s speech. (Ok, cue the tears) Do you hear what I am saying? Out of everything my daughter excitedly tries to share with me, I miss up to half of it most of the time.

This past spring she had additional surgery to fix her pharyngeal flap. This flap closes off the airway between her nose and throat. Speech ordered the surgery to help with her nasal emissions. It’s helped some but it’s still an uphill battle given her poorly aligned jaw and all the air it allows through when she attempts certain sounds. And she’s trained her vocal chords to compensate for the lack of a pharyngeal flap. But you know what? She can say “s” now. Perfectly. I can tell it apart from an “F.” And she can blow up a balloon, blow bubbles, and clearly say “Please Push me mommy!” Most importantly, she can now clearly tell me I am STILL her best friend.

While in the NICU she underwent genetic testing to locate a potential cause or additional factors for her isolated bilateral complete soft and hard palate (that means there was NO palate up there on either side, hard or soft. Hard is when you put your tongue straight up. Soft is when you slide it back towards your throat.) But there was no additional genetic reason. She was diagnosed with isolated Pierre Robin Sequence. This more often than not occurs in boys, not girls. We hit the jackpot.

Despite all of her hardships, all of her struggles, all of the things she will face as she grows and has to overcome new challenges and issues, Charlotte is one of the happiest people I have ever met. Her goal in life? To make us smile and laugh. All the time. For real (to steal a phrase from her).

I went through hell with her. Emotionally, physically, every kind of -ally you can think of. I was there. Gripped on for dear life. Looking back, I know I was depressed during my pregnancy with her. Weighed down with an unresolved postpartum depression from my first pregnancy. Cleft defects often happen within the first 6-8 weeks of pregnancy. Before I even knew I was pregnant our angel was already awaiting us with a huge surprise all her own.

Where am I going with all of this?

I’m filling you in on how hard it is to be a NICU parent of a premature baby. What kind of challenges we face. How it doesn’t all end when we step out of the sterile nursery where our children spent their first days, months, or possibly year. We worry when things come up – anything – about it being related to something that happened at birth – is this because of such and such? She’s got a cold. How will this affect her airway? Should I let her sleep on her back if she’s so congested? What if she stops breathing? And so the monster is fed.

But on the flip side I am truly amazed at how often I manage to deny the monster his food. How often I am able to keep a cool head and maneuver my way around the big issues. I remember time B.C. (Before Charlotte). I would watch shows filled with parents of special needs kids. Amazed I would wonder where on earth they found the emotional stamina to wake up in the morning and face another day knowing the challenges that lay in store. But they did and I do. I look forward to her giggles. I look forward to her playful eyes, her hugs, her kisses, her tantrums, her excited babbling when her bus is about to arrive. And sometimes I want to cry. But mostly I want to rejoice. God has gifted me a perfect Angel and one day, in HIS time, I will understand her perfectly.

Tomorrow we go to Atlanta with Charlotte for follow up with the Cleft Palate Clinic.

I would be lying if I said I was not nervous.

This appointment was supposed to have taken place when she was nine months old.

She’ll be three years old next month.

Charlotte in the NICU

It took me this long to get to the point where I could even think about facing the hospital where she spent her first 21 days of life without having an anxiety attack.

This is the same hospital in which I tucked myself into a corner of the sleep room in the NICU area, blasted Linkin Park over the MP3 player and checked out. No desire to come back. Just wanted to stay curled up under the blanket and pretend none of this was happening. Nope. Not to me. I didn’t have a baby in the NICU. She wasn’t downstairs having major jaw surgery at just nine days old. We weren’t doing this. I was stuck in the middle of a really bad dream and I’d wake up at home with a normal baby.

I can still see that hallway, that sleep room, my nostrils fill with the scent of the surgical soap that killed my hands as I washed them every time we went into the NICU, every time i pumped, every time I went to the restroom there.

I remember the pumping rooms in which I spent most of my time staring at the clock wishing I could nurse my daughter instead of shoving my breasts into hard cold flanges, flicking a switch on a massive antique pump, adjusting the suction to just below Holy Crap that Friggin Hurts.

But tomorrow is the day we finally go back.

Chris is going with me as a safety. I don’t know how I will handle this. I’m hoping for the best. Praying for the best. I keep thinking about how far we’ve come since then and how lucky we are that we don’t have a lot of the problems a lot of parents have with their Pierre Robin kids. She’s talking, using sentences nonetheless. She’s breathing on her own. She eats – oh lord, she eats – she’d eat herself sick (and has) if we let her. No oral aversions here.

But she does have a fistula – an opening in her palate repair. It’s at the back of the throat. And her enunciation is off – it’s nasal. She can’t say “s” without blowing air through her nose. Chris and I understand maybe 75 – 80% of what she says and it breaks our hearts that we can’t even understand our own child all the time. It’s led to frustration on both sides and is now turning into a discipline issue.

I’m afraid we’ll be told she needs surgery. I’m afraid of what that will mean for us and for her. I’ve talked with her about the possibility of surgery. She knows that they would give her some medicine to help her go to sleep and fix her mouth while she was asleep. That she might be owwwy when she wakes up and that they’d have medicine ready to help with the owwwy.

She seems cool with it.

I’m not.

I have forgotten how to let her go with the doctors – I got so good at it when she was in the NICU but she’s been all ours for almost three years now. I don’t want to hand her over to be taken to surgery. I want to go with her! That’s my baby you’re taking!

But now I’m thinking too much and need to stop and let God do all this worrying for me.

Please pray for us as we face tomorrow.

Pray for a peaceful heart and soul for me.

Pray for a pain-free and comfortable day for Chris as he goes with us.

Pray for a positive evaluation.

Pray that I am able to handle any news of surgery with strength and grace and truly give it to God.

I know that I’ve mentioned our second daughter was born with a cleft palate and spent some time in the NICU. While she is now free of most artificial attachments (she still has tubes in her ears), every day is a new day with her – she will be 18 months old this month and is still not speaking. Babbling, yes, but not talking. I also still have to feed her because if we let her feed herself, she will eat entirely too much and gag/choke. I also have to balance the fine line between too much food and not enough food. She is a bottomless pit, something I attribute to her being primarily tube fed for the first six months of her life. And this is beyond the normal baby care. Alot of mothers do more, and a lot do less. But for me, it’s my new normal. I check her mouth whenever she is teething to make sure there’s not one popping through the roof of her mouth.

All that being said, I still belong to an email group for parents of children with PRS (Pierre Robin Sequence/syndrome). Today, one of the moms sent a precious email. I will warn you – it made me cry. And I am not a crier. This one really hit home with me and I needed to read it. I LOVE how things like this that you need to read pop up right when you need them to!

So this is for mothers of handicapped/special needs kids – Know that you are amazing.

Some women become mothers by accident, some by choice, a few by social pressure and a couple by habit.This year, nearly 100,000 women will become mothers of handicapped children. Did you ever wonder how mothers of handicapped children are chosen? Somehow I visualize God hovering over earth selecting His instruments for propagation with great care and deliberation.As He observes, He instructs His angels to make notes in a giant ledger. “Armstrong, Beth, son… Patron Saint, Matthew” “Forrest, Marjorie, daughter… Patron Saint, Cecillia” “Rudledge, Karen, Twins… Patron Saint… give her Gerard, he is used to profanity.”

Finally, He passes a name to an angel and smiles, “Give her a handicapped child.” The angel is curious, “Why this one, God? She is so happy.” “Exactly,” smiled God. “Could I give a handicapped child to a mother who does not know laughter? That would be cruel.” “But has she got patience?” asked the angel. “I don’t want her to have too much patience or she will drown in a sea of self-pity and despair. Once the shock and resentment wear off, she’ll handle it. I watched her today. She has that feeling of self and independence that is so rare and so necessary in a mother.

You see, the child I am going to give her has his own world. She has to make the child live in her world and that is not going to be easy.” “But Lord, I don’t think she even believes in You.” God smiled, “No matter. I can fix that. This one is perfect. She has just enough selfishness.” The angel gasped, “Selfishness? Is that a virtue?” God nods. “If she can’t seperate herself from the child ocassionally, she will never survive. Yes, here is a woman I will bless with a child less than perfect. She doesn’t realize it yet, but she is to be envied. She will never take for granted a “spoken word.” She will never consider a “step” ordinary. When her child says “Momma,” for the first time, she will be present at a miracle and know it! When she describes a tree or sunset to a blind child, she will see it as few people ever see my creation. I will permit her to see clearly the things I see… ignorance, cruelty, prejudice… and allow her to rise above them. She will never be alone. I will be at her side every minute of every day of her life because she is doing My work as surely as if she were here by My side.” “And what about her Patron Saint?” asked the angel, his pen poised in mid air. God smiles, “A mirror will suffice.”

A Note

Please note any information found on this blog is not meant to replace that of a qualified professional.
We encourage partnership with your physician, psychiatrist, and therapist in the treatment of mood disorder.
The information found here is educational and anecdotal and should be reviewed with a professional prior to implementation.